Moyamoya disease is a progressive cerebral arteriopathy with distal ICA stenosis/occlusion on angiography and an extensive network of collateral vessels secondary to chronic cerebral ischemia. Interventions halting progression of perfusion changes are limited to direct or indirect neurosurgical revascularization procedures and antiplatelet medications. The roles of hyperbaric oxygen and induced hypertension in the setting of poor perfusion are not well described.

We reviewed laboratory data and imaging studies of a patient with recurrent ischemic strokes diagnosed with Moyamoya disease.

A 33 year-old female was admitted for progressive multifocal ischemic infarcts with improving left facial droop and mild right-sided weakness (NIHSS improved from 5 to 1 on presentation).  She initially underwent cerebral angiogram, revealing bilateral ICA stenosis with loss of bilateral MCA filling, consistent with Moyamoya disease.  She was placed on aspirin with plan for outpatient EDAS procedure.  Prior to discharge, she developed acute neurologic decline with severe aphasia and worsening right-sided weakness (NIHSS score increased to 14),  with new bilateral MCA territory core infarcts and significant mismatch on CT perfusion. Therapeutic hypertension was induced with vasoactive medications to increase MAP and CPP. She also underwent 10 days of hyperbaric oxygen therapy. Interval imaging demonstrated improved perfusion without core infarct progression.  She underwent tracheostomy and PEG placement and was discharged on mono-antiplatelet therapy with plan for potential EDAS once medically stable.

Hyperbaric oxygen and hypertension hold potential therapeutic benefit in Moyamoya disease complicated by recurrent cerebral ischemia. Our patient demonstrated a stable perfusion profile after a new ischemic event with prompt induction of hypertension with an elevated MAP and 10 days of hyperbaric oxygen therapy. Further studies are needed to determine the definitive risks and benefits of these interventions and the potential for early intervention to prevent worsening ischemia.